Abstract Background/Aims Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is an uncommon clinical triad often posing significant diagnostic challenges. Pathophysiology is attributed to the release and systemic circulation of pancreatic enzymes, such as amylase and lipase, which cause saponification and necrosis of distant adipose tissue, leading to the characteristic musculoskeletal and dermatological manifestations. Methods A 79-year-old female with a history of excessive alcohol consumption presented with acute onset pain and swelling in both knees and ankles, significantly limiting her mobility. Prior to the admission, she developed a painful, red, blotchy, nodular rash extending to her mid-thighs. Following admission, she complained of epigastric abdominal pain and was subsequently diagnosed with acute pancreatitis. Examination identified B/L knee and ankle arthritis and resolving erythematous nodular skin lesions on her lower limbs. Her rash improved quite rapidly during her inpatient stay, hence a skin biopsy was deferred. Investigations revealed markedly elevated serum amylase (603) and CRP (256), with a normal WBC; weakly positive antinuclear antibody (ANA); negative ENA, rheumatoid factor (RF), and anti-cyclic citrullinated peptide (anti-CCP). CT scan of the pancreas confirmed acute pancreatitis. Although a plain radiograph of the knee demonstrated chondrocalcinosis, aspiration of the right knee joint fluid showed no evidence of crystals or infection. Initially, a diagnosis of crystal arthritis was considered, and treatment with colchicine was commenced. However, this was discontinued after four days due to gastrointestinal intolerance and without any significant improvement. MRI of the knee was pivotal, identifying intramedullary fat necrosis in the proximal tibia consistent with PPP syndrome. The final diagnosis was acute pancreatitis-associated panniculitis and intramedullary fat necrosis. Her care was managed by a multidisciplinary team including the alcohol care, gastroenterology, and rheumatology services. Treatment consisted of supportive measures for pancreatitis, including intravenous fluids, thiamine, analgesia, and antiemetics, leading to gradual resolution of polyarthritis and improvement in pancreatitis. She was discharged. Results The diagnosis of PPP syndrome can be challenging due to its rarity and its mimicry of more common inflammatory and infective conditions. A high index of suspicion is required, particularly in patients with a history of recurrent pancreatitis or chronic alcoholism presenting with arthritis and skin lesions. This condition’s prognosis is varied; mortality is reported to be up to 24%, primarily linked to underlying pancreatitis. Conclusion Recognition of PPP syndrome is essential for clinicians to avoid misdiagnosis and the potentially harmful administration of inappropriate immunosuppressive therapy. The management of the underlying pancreatitis typically leads to the resolution of the systemic manifestations. This case vividly highlights the characteristic clinical triad and the importance of detailed imaging and close collaboration with radiology. Disclosure R. Younus: None. F. Zain: None. E. Smith: None. M. Soliman: None. A.M. Desai: None.
Younus et al. (Wed,) studied this question.